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Renal 2 > Robbins Ch. 20 - Cysts, Obstruction, Neoplasia > Flashcards

Robbins Ch. 20 - Cysts, Obstruction, Neoplasia Flashcards

1
Q

Kidney agenesis must be ____ to survive

What else will you see?

A

UNILATERAL

Compensatory enlarging of solitary kidney

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2
Q

Most common site of ectopic kidney?

Complication?

A

Pelvic brim or within pelvis

Ureteral kinking/tortuosity –> obstructed flow

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3
Q

Locations of renal colic

A

12th rib costovertebral angle (CVA) to groin

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4
Q

Unilateral cystic, enlarged kidney
Abdominal mass on palpation
Undifferentiated mesenchyme pockets with cartilage
Other GU abnormality (missing ureter, obstruction, etc.)

A

Multicystic renal dysplasia

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5
Q

Bilateral enlarged, multicystic kidneys
Hematuria –> proteinuria, polyuria, HTN
Adult

A

Autosomal dominant (Adult) polycystic kidney disease

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6
Q

ADPKD patients can often have what other congenital anomalies?

A

Liver cysts, berry aneurysms, mitral valve prolapse, HTN coronary/heart disease

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7
Q

ADPKD genetics

A

PKD1 (polycystin-1) or PKD2 (polycystin-2) gene mutations

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8
Q

Enlarged, smooth, sponge-like kidneys

Neonate with kidney failure

A

Autosomal recessive (childhood) polycystic kidney disease

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9
Q

ARPKD patients often have what else?

A

Portal and systemic HTN, hepatic fibrosis

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10
Q

ARPKD genetics

A

PKHD1 gene deficiency (fibrocystin)

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11
Q 
Cysts at the corticomedullary junction
Shrunken kidneys
Chronic tubulointerstitial nephritis
Child
Polyuria, polydipsia
Growth retardation
A

Familial juvenile nephronophthisis

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12
Q

Genetics of FJN

A

Autosomal recessive - NPHP gene mutations

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13
Q

Expected outcome for FJN

A

ESRD in 5-10 years

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14
Q 
Cysts at the corticomedullary junction
Shrunken kidneys
Chronic tubulointerstitial nephritis
Adult
Polyuria, polydipsia
A

Adult-onset nephronophthisis

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15
Q

Genetics of adult-onset nephronophthisis

A

Autosomal dominant - MCKD gene mutations

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16
Q

Expected outcome for adult-onset nephronophthisis

A

Chronic renal failure

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17
Q

Only medullary cystic disease WITHOUT an inheritence pattern

A

Medullary sponge kidney

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18
Q

Adult
Normal renal function
Collecting ducts are dilated (grossly)
Small cysts lined with epithelium in medulla

A

Medullary sponge kidney

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19
Q

Patient on chronic dialysis

Cortical and medullary cysts/stones

A

Acquired cystic disease (dialysis-associated)

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20
Q

Acquired cystic disease:

Renal stones made of ____
Risk for developing ____

A

Calcium oxalate

Renal cell carcinoma

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21
Q

Microscopic hematuria
Cortical cyst(s)
Normal-sized kidneys

Clinical outcome?

A

Simple cysts

Benign

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22
Q

Teenager/young adult with GENETIC form of ESRD…

Most likely to be what?

A

Familial/juvenile nephronophthisis

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23
Q

Obstructive lesions increase risk of ____

A

Infection and stone formation

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24
Q

Chronic unrelieved obstruction leads to ____

A

Renal atrophy (Hydronephrosis, Obstructive uropathy)

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25
Hydronephrosis
Dilation of renal pelvis and calyces due to atrophy of kidney due to urine outflow obstruction
26
Renal colic (pain in lower thoracic and lumber) Bladder symptoms Distention of collecting system
Acute obstruction
27
``` Urinates a lot Urinates at night Hypertension Renal calculi Tubulointerstitial nephritis ```
Bilateral partial obstruction
28
Male, 20s, intense unilateral renal colic | Increased urinary concentration of certain molecule (supersaturation of it)
Nephrolithiasis
29
Causes of calcium oxalate stone formation
Hypercalciuria via... - Hypercalcemia - Hyperparathyroidism - Diffuse bone disease - Hyperabsorption by intestine - Sarcoidosis - Idiopathic hypercalciuria (w/o hypercalcemia)
30
Bacterial infection Large renal stones in renal pelvis Most likely bacteria? (3)
Magnesium ammonium phosphate stones Urea-splitters (Proteus, Klebsiella, Staph)
31
Does having uric acid stones indicate hyperuricemia or excess excretion of uric acid?
NOT ALWAYS -
32
4 influencing factors for calculi formation
- Increased concentration of constituents - Urinary pH change - Decreased urinary volume - Bacterial infection
33
Most common renal neoplasm
Renal papillary adenoma
34
Small, well-circumscribed nodule in the renal cortex made of complex branching with papillary fronds. Found to be of epithelial origin. Prognosis? Genetics?
Renal papillary adenoma Benign (if less than 3 cm) Trisomy 7 or 17
35
Adult LARGE (12 cm) renal tumor with large eosinophilic cells w/ numerous mitochondria Tan/brown, well-encapsulated, central scar Important differential Dx? Malignant?
Oncocytoma Non-clear cell renal cell carcinoma Benign
36
Adult female (Retroperitoneal hemorrhage, shock) Renal tumor with vessels, muscle, and fat Skin abnormalities, neurologic symptoms, other tumors (heart, etc.) Most likely genetic mutation? Hereditary pattern?
Angiomyolipoma TSC1 or TSC2 (TUBEROUS SCLEROSIS) - Autosomal dominant
37
Easy way to know an angiomyolipoma on pathology presentation
``` Thick blood vessels ("angio") Smooth muscle ("myo") Fat vesicles ("lipoma") ```
38
Older adult, smoker Costovertebral pain Palpable renal mass Hematuria
Renal cell carcinoma
39
Familial forms of renal cell carcinoma
- Von Hippel-Lindau - Hereditary leiomyomatosis/renal cell cancer - Hereditary papillary carcinoma - Birt-Hogg-Dubé syndrome
40
Biggest risk factor for renal cell carcinoma
TOBACCO
41
Non-papillary unilateral unifocal renal tumor Chr. 3 (VHL) deletion Proximal tubular epithelium
Clear cell carcinoma
42
Pathophysiology of clear cell carcinoma
Low VHL = high HIF-1 = high VEGF, IGF-1
43
Papillary multifocal renal tumors Trisomy 7, 17 Mutated (active) MET gene
Papillary carcinoma
44
Renal tumor w/ pale eosinophilic cytoplasm and nuclear halos Similar to oncocytoma Prognosis?
Chromophobe renal carcinoma GOOD prognosis (compared to clear cell and papillary)
45
Clear cell carcinomas have mutations on what chromosome?
Chr. 3
46
Papillary cell carcinomas have mutations on what chromosome?
Chr. 7
47
Genetic difference between sporadic and hereditary papillary renal cell carcinomas
Sporadic - Trisomy 17 + loss of Y chromosome ALSO
48
Xp11.2 translocation (TFE3 gene) | Clear cytoplasm, papillary architecture
Xp11 translocation carcinoma
49
RCC metastasizes where?
Renal vein, lung, bone, other
50
Systemic importance of RCC
Many paraneoplastic syndromes of metastases (polycythemia, hyperCa++, HTN, sexual changes, cushings, eosinophilia, amyloidosis, etc.)
51
Rate RCCs by prognosis (6 total)
Good: papillary and chromophobe OK: clear cell Bad: collecting duct, sarcomatoid, medullary
52
Typical location of RCC within kidney
Upper pole
53
Diagnosing clear cell carcinoma
Positive Oil Red-O stain
54
Why are clear cells clear?
Glycogen and LIPID elaboration
55
RCC, spindle cells (like mesenchymal)
Sarcomatoid RCC
56
RCC, branching tubules w/ cuboidal cells
Collecting duct carcinoma
57
RCC, sickle cell disease
Medullary carcinoma
58
Gross hematuria Palpable hydronephrosis Flank pain Smoker Most likely locations?
Urothelial (transitional cell) carcinoma Renal pelvis AND BLADDER
59
Prognosis of urothelila carcinoma
Bad
60
4 most common childhood MALIGNANT tumors
1. Acute leukemia 2. Neuroblastoma 3. Retinoblastoma 4. Wilms tumor
61
Child, 2-5 y/o Large abdominal mass (unilateral usually) Pain, microscopic hematuria, HTN Metastatic
Wilms tumor (nephroblastoma)
62
Familial syndromes w/ wilms tumor
WAGR (wilms-aniridia-genital-retardation) Denys-drash syndrome (gonadal and renal) Beckwith-Wiedemann syndrome
63
Percentage of wilms tumors that are congenital/genetic
10%
64
WAGR and DDS have what mutation?
Chr. 11 (WT1 gene)
65
B-W syndrome has what mutation?
None or WT2
66
Wilms tumor | Hypertrophy of one half of body
Beckwith-Wiedemann syndrome
67
Wilms tumor Mental retardation No colored portion of eye
WAGR syndrome
68
Wilms tumor | Gonadal tumor
Denys-Drash syndrome
69
Wilms tumor + nephrogenic rests --> suspect what?
BILATERAL + MULTIPLE tumors
70
2 morphologies of wilms tumor
- Triphasic (blastema, epithelial (tubular), stromal) | - Anaplastic (pleomorphism and atypia)
71
Anaplastic wilms tumor... | - 2 things to note
1. p53 mutation | 2. Resistant to chemo
72
Location and morphology of wilms tumor
Lower pole Tan/gray color Well-circumscribed
73
Prognosis of wilms tumor
GOOD - curable
74
How to genetics and age relate to prognosis of wilms tumor?
p53 (anaplastic), gene mutations = WORSE | Younger age = WORSE
75
Most common metastases TO the kidneys Locations?
Small cell lung Bilateral, multifocal

Renal 2 (11 decks)

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